J Rheum Dis.  2024 Apr;31(2):116-119. 10.4078/jrd.2023.0052.

Lupus nephritis presenting with massive ascites and pleural effusion (pseudo-pseudo Meigs’ syndrome)

• Deniz R
• Hacımurtazaoğlu-Demir G
• Karaalioğlu B
• Özgür DS
• Akkuzu G
• Yıldırım F
• Bes C

• KMID: 2554360
• DOI: http://doi.org/10.4078/jrd.2023.0052

Abstract

The triad of ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) levels in the absence of ovarian malignancy in systemic lupus erythematosus patients is specifically named pseudo-pseudo Meigs’ syndrome (PPMS) or Tjalma syndrome. In this case we reported a 33 years female patient with pleural effusion lasting for 3 years and new onset progressive massive ascites and increased level of CA-125. After she was evaluated for an underlying benign and malign ovarian tumor or any other malignancies, serologic tests were requested with respect to progressive renal dysfunction, proteinuria, lymphopenia, anemia, and effusion. She was diagnosed with systemic lupus erythamatosus (SLE) and renal biopsy showed class-V lupus nephritis. Immunosuppressive treatment led to improvement in both SLE activity and components of PPMS, including massive ascites and pleural effusion and without the need of diuretics. Co-existence of unexplained CA-125 increase, pleural effusion, and ascites might be related to PPMS and detailed examination to exclude malignancy and early and effective treatment of SLE are the mainstay of management.

Keyword

Systemic lupus erythematosus; Lupus nephritis

Figure

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  Figure 1 (A) Clinical appearance of massive ascites in patient. (B) Computed tomography scan of the abdomen showing abundant free liquid in the abdomen as massive ascites with normal bilateral ovaries, increased number and engorgement of mesenteric vessels exhibiting comb sign.

Reference

1. Tjalma WA. 2005; Ascites, pleural effusion, and CA 125 elevation in an SLE patient, either a Tjalma syndrome or, due to the migrated Filshie clips, a pseudo-Meigs syndrome. Gynecol Oncol. 97:288–91. DOI: 10.1016/j.ygyno.2004.12.022. PMID: 15790480.

2. Widra EA, Armstrong J. 1995; Pseudo-Meigs' syndrome and lupus. Int J Gynaecol Obstet. 49:193–4. DOI: 10.1016/0020-7292(95)02355-G. PMID: 7649329.

3. Meena DS, Kumar B, Gopalakrishnan M, Kachhwaha A, Kumar S, Sureka B, et al. 2021; Pseudo-pseudo Meigs' syndrome (PPMS) in chronic lupus peritonitis: a case report with review of literature. Mod Rheumatol Case Rep. 5:300–5. DOI: 10.1080/24725625.2021.1916160. PMID: 33970813.

4. Quintero-Muñoz E, Gómez Pineda MA, Araque Parra C, Vallejo Castillo CA, Ortega Marrugo V, Bonilla Jassir J, et al. 2021; Is there any relationship between massive ascites and elevated CA-125 in systemic lupus erythematosus? Case report and review of the literature. Mod Rheumatol Case Rep. 5:292–9. DOI: 10.1080/24725625.2021.1909213. PMID: 33783326.

5. Thaker DA, Stride PJ, Dettrick A. 2011; A pseudo case of atypical pseudo-Meigs syndrome. Indian J Cancer. 48:364–6. DOI: 10.4103/0019-509X.84914. PMID: 21921340.

6. Wiatrowska B, Krajci P, Berner A. 2000; [Pseudo-Meigs' syndrome]. Tidsskr Nor Laegeforen. 120:364–6. Norwegian.

7. Bes C, Dağlı Ü, Memedoğlu P, Soy M. 2013; A rare form of SLE: pseudo-pseudo meigs syndrome and hydrocephalus. Rheumatol Int. 33:2175–6. DOI: 10.1007/s00296-012-2420-6. PMID: 22451030.

8. Bes C, Soy M. 2011; Pseudo-pseudo Meigs syndrome developed under the leflunomide therapy. Rheumatol Int. 31:521–3. DOI: 10.1007/s00296-009-1190-2. PMID: 19844717.

9. Chao YH, Chen HY. 2022; Rare cause of ascites and pleural effusion: the first case report and literature review of pseudo-pseudo Meig's syndrome in Taiwan. J Formos Med Assoc. 121:2633–8. DOI: 10.1016/j.jfma.2022.03.020. PMID: 35459608.

10. Torres Jiménez AR, Solís-Vallejo E, Céspedes-Cruz AI, Zeferino Cruz M, Rojas-Curiel EZ, Sánchez-Jara B. 2019; Tjalma syndrome (pseudo-pseudo Meigs') as initial manifestation of juvenile-onset systemic lupus erythematosus. Reumatol Clin (Engl Ed). 15:e41–3. DOI: 10.1016/j.reumae.2018.09.003.